The authors present the case of a 43–year–old Caucasian man with advanced chromophobe renal cell carcinoma treated with the tyrosine kinase inhibitor sunitinib and subsequently with sorafenib and the mammalian target of the rapamycin inhibitor everolimus, achieving a prolonged response and significant clinical benefit. The authors report an unexpectedly high efficacy of everolimus as a third–line treatment in a patient with metastatic chromophobe renal cell carcinoma.